Ann Thorac Surg 2005;80:1515-1517
© 2005 The Society of Thoracic Surgeons
Case report
Primary Extramedullary Hematopoiesis Manifesting As Massive Bilateral Chylothorax
Arup K. Ghosh, MS, FRCS
a
,
Joya Pawade, MD
b
,
Graham R. Standen, FRCP
c
,
Loïc Lang-Lazdunski, MD, PhD
a
,
*
a Department of Thoracic Surgery, Bristol, United Kingdom
b Department of Pathology, Bristol, United Kingdom
c Department of Hematology, Bristol Royal Infirmary, Bristol, United Kingdom
Accepted for publication March 30, 2004.
* Address reprint requests to Dr Lang-Lazdunski, Department of Cardiothoracic Surgery, Guy's Hospital, St. Thomas St, London SE1 9RT, UK (Email: loic.lang-lazdunski{at}gstt.nhs.uk).
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Abstract
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Intrathoracic extramedullary hematopoiesis is a rare entity, mostly observed in patients with hematologic disorders. Extramedullary hematopoiesis is usually asymptomatic and is often located in the lower paravertebral sulci and rarely in the pleura. We report the case of a 54-year-old man without hematologic disorder or pleural malignancy who had a massive bilateral chylothorax develop due to primary pleural extramedullary hematopoiesis. He was successfully treated by bilateral video thoracoscopic talc pleurodesis and low-dose radiotherapy.
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Introduction
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Extramedullary hematopoiesis (EMH) occurs as a compensatory mechanism to several hematologic disorders such as myelofibrosis, thalassemia, and sickle cell anemia [1]. Extramedullary hematopoiesis is a rare entity that is usually asymptomatic and generally diagnosed on postmortem examination, which has been reported to occur as fibrous hematopoietic tumors in the mediastinum or pleura. This has also been associated with hemothoraces or sanguineous pleural effusion [1–8]. We report the case of a patient who presented with massive bilateral chylothorax and was subsequently diagnosed as having symptomatic diffuse pleural EMH.
A 54-year-old man without previous significant medical history was referred to our department for the exploration and treatment of a bilateral chylothorax. He had been admitted at another hospital with progressive breathlessness. He denied any weight loss or night sweats. He was a nonsmoker without asbestos exposure. Chest roentgenogram and computed tomography revealed a massive bilateral effusion (Fig 1). Chest and abdominal computed tomographic scans revealed neither mediastinal nor retroperitoneal lymphadenopathy. His liver and spleen were of normal dimension. Physical examination revealed a blood pressure of 150/90 mm Hg and a pulse rate of 86 per minute. His oxygen saturation on air was 94%. Blood tests revealed a hemoglobin level of 15.2 g/dL, hematocrit value of 44.6%, mean corpuscular volume of 83 fL, mean corpuscular hemoglobin concentration (MCHC) of 37.1 g/L, red blood cell count of 5.38.106/mm3, white blood cell count of 7.10.103/mm3, and platelet count of 377.103/mm3. His lactate dehydrogenase level was 298 IU/L. His C-reactive protein level was less than 10 mg/L. A thoracentesis yielded a sanguineous-chylous effusion with a pH of 7.46, a glucose of 5.6 mmol/L, and triglycerides of 9.2 mmol/L. Cytology disclosed isolated megakaryocytes.
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Fig 1. Preoperative chest computed tomographic scan demonstrates massive bilateral pleural effusion without evident pleural thickening. Neither pulmonary nor mediastinal mass nor lymphadenopathy is visible.
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Considering the potential for pleural malignancy, the patient was referred to us for a video thoracoscopic pleural biopsy.
Bilateral video thoracoscopy disclosed a large amount of sanguineous-chylous effusion (3 L aspirated on each side). There was no pleural or mediastinal tumor on either side. At operation, the surgeon noted a spontaneous chylous leak from the mediastinal pleura, bilaterally. No attempt was made to biopsy the mediastinal tissue considering the risk of severe hemorrhage. Several biopsies were taken in the lateral and posterior parietal pleura, and a lung biopsy was taken from the right lower lobe. Bilateral talc insufflation was performed and bilateral drains were inserted.
Pathology revealed foci of extramedullary hematopoiesis characterized by the presence of megakaryocytes, erythroid colonies, and myeloid colonies in the pleura (Fig 2). All three lineages were mixed and showed no evidence of maturation. No dysplastic features were seen. There was neither abnormal pulmonary hematopoiesis nor pulmonary lymphangiectasia.
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Fig 2. Pleural histopathology of solitary megakaryocyte surrounded by other hematopoietic colonies in the parietal pleura.
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The treatment consisted of bowel rest and total parenteral nutrition for nutritional support. His postoperative course was uneventful. Chest drains were removed after 4 days on the left side and after 14 days on the right side. An iliac crest bone marrow biopsy was performed in a systematic way, but disclosed no evident hematologic disorder. The patient was discharged home after 25 days, but was readmitted 10 days later with a recurrent bilateral chylothorax. A new chest drain was inserted on the right side, and the patient was given low-dose radiation therapy (2 Gy in 4 fractions). There has been no recurrence of pleural effusion to date, and the patient's condition has improved dramatically. He is currently working full time 11 months postoperatively.
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Comment
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Extramedullary hematopoiesis has been reported to occur in patients with hematologic disorders such as a fibrous hematopoietic tumor in the pelvis, mesentery, retroperitoneum, mediastinum, pleura, and lung [1]. Rarely, EMH may cause neurologic symptoms due to epidural localization leading to spinal cord or cauda equina compression [1].
Various noninvasive procedures have been advocated to establish the diagnosis of intrathoracic EMH. These include contrast-enhanced computed tomography, magnetic resonance imaging, technetium-99 radionuclide bone marrow scanning, cytologic study of the pleural fluid, and fine needle aspiration of the thoracic masses [1]. Video thoracoscopy has been useful in some patients with recurrent pleural effusion for performing pleural biopsy and associated pleurodesis [1, 4, 5].
Treatment of EMH is usually unnecessary in the absence of complication. Only a few cases of complicated intrathoracic EMH have been reported in the literature [1–8]. Smith and colleagues [7] reported a patient with thalassemia who had a hemothorax develop, which was successfully treated with radiation therapy to prevent recurrent bleeding. Oren and colleagues [2] and Bartlett and colleagues [5] also reported patients who were successfully treated with low-dose radiation therapy. Although tetracycline pleurodesis has been successfully attempted in a patient with intrathoracic EMH and recurrent pleural effusions [5], Kupferschmid and colleagues [6] reported a patient with a massive hemothorax in whom tetracycline accelerated pleural bleeding. Hemothorax was ultimately controlled with low-dose radiation therapy. Ibabao and colleagues [3] reported a patient with mediastinal masses who was successfully treated by bilateral talc pleurodesis. Muthuswamy and colleagues [8] reported a patient who suffered a massive hemothorax due to intrathoracic EMH after sustaining chest trauma.
In our patient, the importance of chylothorax prompted us to perform early video thoracoscopy for pleural biopsy. Extramedullary hematopoiesis was not suspected preoperatively, because the patient had no evidence of hematologic disorder and neither chest computed tomography nor abdominal computed tomography revealed spleen or liver enlargement nor pleural or mediastinal mass. The operative findings led us to perform a bilateral talc pleurodesis to prevent recurrence. As bone marrow trephine biopsy disclosed no hematologic disorder, it was decided to discharge the patient without adjuvant radiation. Early recurrence of chylothorax prompted us to give the patient low-dose radiotherapy to the mediastinum and pleura, although no mass or hematologic disorder had been diagnosed. We believe that our present case report is the first case of primary intrathoracic EMH without underlying hematologic disorder. We also believe this is the first reported case of chylothorax associated with EMH.
Therefore we speculate that diffuse pleural and mediastinal EMH may have impaired chylous drainage from the pleura and was responsible for the massive chylothorax observed in our patient. Although chest tube insertion and thoracoscopic pleurodesis should be considered as first line therapy in patients with EMH and symptomatic pleural effusion, our report emphasizes the role of low-dose radiotherapy as a second-line therapy in patients with refractory pleural effusions.
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References
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- Chu KA, Lai RS, Lee CH, Lu JY, Chang HC, Chiang HT. Intrathoracic extramedullary haematopoiesis complicated by massive haemothorax in alpha-thalassemia Thorax 1999;54:466-468.[Abstract/Free Full Text]
- Oren I, Goldman A, Haddad N, Azzam Z, Krivoy N, Alroy G. Ascites and pleural effusion secondary to extramedullary hematopoiesis Am J Med Sci 1999;318:286-288.[Medline]
- Ibabao J, Kassapidis S, Demetis S, et al. Bilateral pleural effusions in a beta-thalassemia intermedia patient with posterior mediastinal extramedullary hematopoietic masses Hemoglobin 1999;23:249-253.[Medline]
- Bartlett RP, Greipp PR, Tefferi A, Cupps RE, Mullan BP, Trastek VF. Extramedullary hematopoiesis manifesting as a symptomatic pleural effusion Mayo Clin Proc 1995;70:1161-1164.[Abstract]
- Peng MJ, Kuo HT, Chang MC. A case of intrathoracic extramedullary hematopoiesis with massive pleural effusionsuccessful pleurodesis with intrapleural minocycline. J Formos Med Assoc 1994;93:445-447.[Medline]
- Kupferschmid JP, Shahian DM, Villanueva AG. Massive hemothorax associated with intrathoracic extramedullary hematopoiesis involving the pleura Chest 1993;103:974-975.[Abstract/Free Full Text]
- Smith PR, Manjoney DL, Teitcher JB, et al. Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with thalassemia intermedia Chest 1988;94:658-660.[Abstract/Free Full Text]
- Muthuswamy PP, Shah P, Patel R, et al. Intrathoracic extramedullary hematopoiesis simulating post-traumatic intrathoracic hemorrhage Am J Med 1989;86:358-360.[Medline]
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Abstract
Introduction
